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Managing Cushing’s disease: the state of the art

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Abstract

Cushing’s disease is a rare chronic disease caused by a pituitary adenoma, which leads to excess secretion of adrenocorticotropic hormone (ACTH). The over-production of ACTH leads to hyperstimulation of the adrenal glands and a chronic excess of cortisol, resulting in the signs and symptoms of a severe clinical state (Cushing’s syndrome) that leads to significant morbidity, negative impacts on the patient’s quality of life, and, if untreated, increased mortality. The management of patients with Cushing’s disease is complicated by the heterogeneity of the condition, with signs and symptoms that overlap with those of other diseases, and high subclinical incidence rates. Controversies surrounding the tests used for screening and identifying patients with Cushing’s disease add to the challenge of patient management. Surgical intervention to remove the adenoma is the first-line treatment for patients with Cushing’s disease, but medical therapies are useful in patients who relapse or are unsuitable for surgery. The recent introduction of pasireotide, the first pituitary-directed medical therapy, expands the number of treatment options available for patients with Cushing’s disease. This state-of-the-art review aims to provide an overview of the most recent scientific research and clinical information regarding Cushing’s disease. Continuing research into improving the diagnosis and treatment of Cushing’s disease will help to optimize patient management.

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Acknowledgments

The authors received writing/editorial support in the preparation of this manuscript provided by Excerpta Medica, funded by Novartis Oncology Region Europe.

Conflict of interest

Dr. Colao has received unrestricted grants from Novartis for research in neuroendocrine and pituitary tumors and is a member of Novartis global and European boards; Dr. Boscaro has nothing to disclose; Dr. Ferone has received unrestricted grants from Novartis for research purposes and is a member of the Novartis advisory boards; Dr. Casanueva has received advisory board fees from Novartis.

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Authors and Affiliations

  1. Dipartimento di Medicina Clinica e Chirurgia, Università Federico II di Napoli, Via S. Pansini 5, 80131, Naples, Italy

    Annamaria Colao

  2. Division of Endocrinology and Metabolism, University Politecnica delle Marche, Ancona, Italy

    Marco Boscaro

  3. Endocrinology, Department of Internal Medicine and Medical Specialties and Center of Excellence for Biomedical Research, IRCCS-AOU San Martino-IST, University of Genoa, Genoa, Italy

    Diego Ferone

  4. Department of Medicine, Santiago de Compostela University, Santiago de Compostela, Spain

    Felipe F. Casanueva

  5. CIBER Obesity and Nutrition, Institute of Health Carlos III, Madrid, Spain

    Felipe F. Casanueva

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  1. Annamaria Colao
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  2. Marco Boscaro
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  3. Diego Ferone
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  4. Felipe F. Casanueva
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Correspondence to Annamaria Colao.

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Colao, A., Boscaro, M., Ferone, D. et al. Managing Cushing’s disease: the state of the art. Endocrine 47, 9–20 (2014). https://doi.org/10.1007/s12020-013-0129-2

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